ALS

Question 1

What are other names for ALS?

Answer 1

• Lou Gehrigs disease

- Maladie de Charcot

Question 2

What is ALS?

Answer 2

Mixed UMN disease

- Involvement of voluntary muscles, sparing the eye muscles & urinary sphincter

Question 3

What is ALS caused by?

Answer 3

Degeneration of neurons in the cervical spine & medulla

- Etiology unknown

Question 4

What is the pathology of ALS?

Answer 4

Degeneration of anterior horn in SC & in brain stem & corticospinal tracts

Question 5

What is the defining feature of ALS?

Answer 5

Both upper & lower motor neurons in the motor cortex of brain, stem, SC

Question 6

What do the motor neurons develop? What does it lead to?

Answer 6

Protein inclusion in the cell –> apoptosis

Question 7

What causes high glutamate levels?

Answer 7

Cell death –> high levels of Ca due to high levels of excitatory neurotransmitters

Question 8

What is an anti-glutamingergic drug?

Answer 8

Riluzole

- The only med tx for ALS

Question 9

How does ALS present?

Answer 9

• Weakness, atrophy of the hands w/ spasticity & hyperreflexia of the lower motor neuro deficits
• Dysarthria & dysphagia
• Tongue atrophy & fasciculations

Question 10

What are some of the “first” sx?

Answer 10

Drop foot
Walking
Speech
Dexterity

Question 11

Bular onset ALS

Answer 11

Speech

Swallow

Question 12

What sx occur in later stages?

Answer 12

Swallowing - aspiration

Intercostal - poor lung fxn

Question 13

How do you dx ALS?

Answer 13

• No definite testing
• UMN & LMN signs
• MRI brain & cervical spine
• EMG
• CSF (protein elevation), HIV, Lyme

Question 14

How do you tx ALS?

Answer 14

• Riluzole (ongoing): inhibits release of gluatmate

- Supportive (tracheostomy, gastrostomy)

Question 15

What is the px of ALS?

Answer 15

Most die within 5 years & are dx around 50-60 years